Association of pheochromocytoma and ganglioneuroma: unusual finding in neurofibromatosis type 1.

OBJECTIVE: To report a rare case of association of pheochromocytoma and ganglioneuroma in an asymptomatic patient with neurofibromatosis type 1 (NF1) and to discuss the importance of annual biochemical and imaging studies. METHODS: We present the clinical, laboratory, and pathology findings in a 41-year-old woman with NF1 and review the pertinent literature. RESULTS: A 41-year-old woman with NF1 presented for a routine gynecologic examination, at which time a right adrenal mass (4 by 3 cm) was discovered by abdominal ultrasonography and confirmed by abdominal computed tomographic scans and magnetic resonance imaging. The patient was normotensive and complained only of discrete essential tremors. Biochemical studies showed a serum epinephrine level of 195 pg/mL (normal, <100) and a 24-hour urine epinephrine excretion of 55 microg (normal, <20), findings consistent with pheochromocytoma. Metaiodobenzylguanidine scintigraphy revealed uptake in the right adrenal gland, with no evidence of metastatic lesions. Before surgical treatment, the patient received an alpha-adrenergic antagonist for 30 days. Laparoscopic excision of the right adrenal gland yielded excellent postoperative results. Surgical pathology revealed a multinodular mass composed of pheochromocytoma and ganglioneuroma. In patients with NF1 (von Recklinghausen's disease), a tumor consisting of pheochromocytoma and ganglioneuroma is rare and may be more aggressive than pheochromocytoma alone. An asymptomatic catecholamine-producing tumor may cause substantial morbidity and mortality, especially in patients who are undergoing surgical intervention or are under other stressors. CONCLUSION: The current guidelines for managing patients with NF1 are an annual history and physical examination. Because of the increased prevalence of pheochromocytoma and ganglioneuroma in patients with NF1, and the potential associated adverse effects, we emphasize the importance of periodic clinical evaluation with biochemical testing and imaging studies.

[Induction of tumors of the sympathetic nervous system in rats]. / Induktsiia opukholei simpaticheskoi nervnoi sistemy u krys.

Benign (ganglioneuromas in 6 rats) and malignant (ganglioneuroblastomas--4) tumors and neuroblastomas (7) were induced in 17 animals by a single dose of 80 mg/kg ethylnitrosourea injected on days 18-19 of gestation. The animals were exposed to continuous lighting during gestation and lactation. Tumors of the sympathetic nervous system were at different stages of maturity. Since most tumors were located along the sympathetic trunk of the mediastinum and retroperitoneal cavity and because of their certain morphological features, such neoplasms may be regarded as an adequate model of similar tumors in children.

Ganglioneuroma of left adrenal gland in a patient with Turner syndrome during growth hormone therapy.

We report on a Japanese girl with Turner syndrome (45,XO) who developed ganglioneuroma of the left adrenal gland during growth hormone (GH) therapy. She had received GH replacement therapy from the age of 6.8 years. At the age of 10.3 years, abdominal ultrasonography revealed a mass which occupied the upper area of her left kidney. Computed tomography and magnetic resonance imaging of the abdomen showed a low density mass with a smooth surface located between the upper portion of the left renal vein and the pancreas. Microscopic examination resulted in a diagnosis of ganglioneuroma of the left adrenal gland. At present we cannot conclude that patients who have received GH replacement therapy are at higher risk for developing tumors compared to those without GH replacement therapy.

Morphology of experimental tumors of the sympathetic nervous system.

The morphology of experimental tumors of the sympathetic nervous system in rabbits and hamsters induced by administration of nitrose compounds and the use of modifying factors were studied. The tumors were localized in the mediastinum, retroperitoneal space, adrenals and kidneys. A predominant involvement of the female animals was observed. Histological, histochemical and electron microscopic studies of 33 tumors of the sympathetic nervous system were carried out. By their degree of maturity the tumors were classified as ganglioneuromas, ganglioneuroblastomas and neuroblastomas. By their structure the experimental tumors were similar to the analogous neoplasias of man.